An Unusual Case of Optic Disc Drusen

An Unusual Case of
Optic Disc Drusen
Shauna Berry, DO
PGY4, Miami, FL
History
14 year-old female presented with a history of
decrease in vision. She states that she has
noticed blurry vision in the right eye for 2
months. She believes her vision is progressively
getting worse. She has no visual complaints in
her left eye. Her last visit was 5 years ago and
she has been seeing a general ophthalmologist
since.
EXAM
•VA: 20/200 with no improvement on PH OD; 20/20 OS
•IOP 16 OD 14 OS
•Pupils: Reactive OU, No APD noted.
•Conj: white and quiet OU
•Iris: Normal OU
•Cornea: Clear OU
•Lens: Clear OU
•Anterior Segment: Deep and quiet OU
•Vitreous: Clear OU
•Optic nerve: optic nerve drusen with a grayish membrane temporal to the optic nerve OD;
Optic nerve drusen OS
•Macula : subretinal hemorrhage nasal, thickening, watermark extends temporal to fovea OD;
Normal foveal light reflex, no subretinal hemorrhage, no subretinal fluid OS.
•Vessels: Normal OU
•Periphery: Retina attached OU
OCT
OCT Retina: Subretinal thickening nasally with mild intraretinal fluid. A small amount of subretinal fluid present OD.
Unremarkable OS.
OCT Nerve: elevation of the optic nerve and hyperreflective deposits OU.
FA
OD: Hyperflouresence temporal to the optic nerve and an
area of blockage nasal to the fovea is present. As the study
progresses there is an increase in hyperflouresence
temporal to the optic nerve consistent with leakage.
OS: Staining of the optic nerve but no leakage in the
macula.
B-Scan
Drusen present OU.
Fundus photography
Fundus Photography
OD: Subretinal thickening and fibrosis through
the nasal macula with a subretinal hemorrhage
nasal to the fovea.
Assessment and Plan
1. Choriodal neovascularization OD
2. Optic nerve drusen OU
Recommend anti-VEGF therapy OD for the choroidal
neovascularization.
Treatment Course
Visit date
Vision OD
Treatment given
Visit Notes
4/10/15
20/200 PH: NI
Initial treatment OD
As noted in previous slides.
5/12/15
20/200 PH 20/160
treatment scheduled for
5/28/15
Subretinal Heme resolved, subretinal
thickening improve. Slight increase in
subretinal fluid. Recommend IVA.
5/28/15
20/200 PH: NI
2nd anti-vegf treatment
OD
Same as above
6/26/15
20/200
PH: 20/160
Pt denies further
treatment.
Definite Improvement from initial visit.
Still some subretinal fluid overlying the
area of subretinal fibrosis. It is unclear if
it will ever resolve. Recommended
additional IVA to complete loading dose.
Fundus Photography: Post tx
Goldman VF OD
A hint of constriction
nasally with slight
enlargement of the blind
spot.
Before & After
Before teatment
Before treatment
After treatment
Before & After
Before teatment
Before treatment
After treatment
Other case reports
•Choriodal neovascularization secondary to optic nerve drusen is rare in children and
literature suggests that further longitudinal studies are warrented in regards to
treatment.
• Gregory Evans, et al. reports a case of a 11 year-old male that presented 20/100 OD
and underwent 3 monthly anti-vegf treatments. VA improved to 20/20. 1
• Alkin, et al. reports a 13 year-old female that presented with 20/80 OD vision. 1
month after 1 anti-vegf treatment VA improved to 20/25 and remained stable for 9
months. Knape report a case of bilateral choridal neovascularization secondary to
optic nerve head drusen. Initial VA was 20/200 OD and 20/70 OS. After the 1st IVA
treatment VA improved to 20/60 OD and 20/70 OS. 2
• Delas, et al. 12 year-old female that presented with CF at 20 cm OD VA. She
underwent two anti-vegf injections and VA improved to 20/60 and remained stable
at 1 year. 3
Optic disc drusen
•
•
•
•
•
•
•
•
•
•
•
Prevalence: 0.34% (clincally) 2% ( on autopsy)
No sex predilection
Often bilateral (75-86%)
Can be sporadic or AD
Theories - impaired ganglion cell axonal transport, probably from small scleral canal
or mechanical obstruction.
Associated with retinitis pigmentosa and psuedoxanthoma elasticum
In children optic disc drusen are generally buried and then become more visible
throughout the years
It is a cause of psuedopapilledema
8.6 % of patients with optic disc drusen have transient visual obscurations
75-87% have a nerve fiber bundle defect.
VF can show an enlarged blind spot, arcuate scotoma, or a sectoral scotoma.
Optic disc drusen
•Can cause serious ocular conditions in the adult population:4
• Central retinal vein occlusion
• Central retinal artery occlusion
• Peripapillary choroidal neovascularization
• Ischemic optic neuritis
• Retinal or optic nerve hemorrhage
•Choroidal neovascularization is a very rare complication in children from optic
disc drusen. The proposed mechanism of the neovascularization is due to a
compressive effect of the drusen on surrounding blood vessels leading to a
compromised vascular integrity, vascular congestion, or ischemia.4
• A study in children with optic disc drusen showed delayed peripapillary
choroidal filling in almost 50 % of the patients.5
Treatment options
•Widely varying treatment options of choroidal neovascularization in children from
optic disc drusen are found within the literature:
• Focal laser photocoagulation
• Photodynamic therapy with verteporfin6
• Surgical removal of the membrane
• Recently Anti-Vegf therapy has been used off-label.
• A combination of focal laser and anti-VEGF has been reported by Knape et al.4
• observation - spontaneous involution has also been reported 7,8
•There are thus far no studies comparing observation with the above reported
treatment modalities.
References
1. Gregory-Evans K, Rai P, Patterson J.” Successful treatment of subretinal neovascularization with intravitreal
ranibizumab in a child with optic nerve head drusen.” J Pediatr Ophthalmol Strabismus 2009. doi:
10.3928/01913913
2. Z Alkin, A Ozkaya, I Yilmaz,A Yazici “A single injection of intravitreal ranibizumab in the treatment of choroidal
neovascularization secondary to optic nerve head drusen in a child” BMJ Case Reports 2014: doi:10.1136/bcr-2014204456
3. Delas B, Almudi L, Carreras A,et al. “Bilateral choroidal neovascularization associated with optic nerve head drusen
treated by antivascular endothelial growth factor therapy.”Clin Ophthalmol 2012;6:225
4. Knape RM, Zavaleta EM, Clark CL III, et al. “Intravitreal bevacizumab treatment of bilateral peripapillary choroidal
neovascularization from optic nerve head drusen.” J AAPOS 2011;15:87
5. Brown S, Del Monte M “Choroidal neovascular membrane associated with optic nerve head drusen in a child.”
American Journal of Ophthalmology. 1995; 121(2): 215-217
6. Torrent R, Loureiro R, Travassos A, et al “ Bilateral CNV associated with optic nerve drusen treated with
photodynamic therapy with verteporfrin.”Eur J Ophthalmol. 2004;14:434
7. M. Munteanu “Hemorrhagic complications of drusen of the optic disc” J Fr Ophthalmology, 30 (1) (2007), pp. 58–67
8. M.J. Harris, S.L. Fine, S.L. Owens .”Hemorrhagic complications of optic nerve drusen.” Am J Ophthalmol, 92 (1)
(1981), pp. 70–76